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1.
Surg Case Rep ; 9(1): 158, 2023 Sep 06.
Article En | MEDLINE | ID: mdl-37672124

BACKGROUND: Synovial sarcoma is an uncommon soft tissue malignancy that mainly occurs near tendon sheath and bone joints. Primary intra-abdominal location is exceedingly rare and characterized by non-specific clinical signs. CASE PRESENTATION: We report the case of a young female without medical history who presented with acute abdominopelvic pain. Ultrasound echography revealed a right mass measuring 7 cm in greater diameter cystic with solid areas, likely of ovarian origin. A coelioscopy with peritoneal biopsies was performed. Histological examination with immunohistochemistry concluded the diagnosis of GIST. The patient was referred to the surgery department and after laboratory routine analysis and computed tomography, the patient was proposed to surgical management. Per-operative findings revealed a mesenteric mass locally invading the greater omentum and the appendicular wall. Pathological examination with immunochemistry confirmed the diagnosis of mesenteric monophasic synovial sarcoma invading the appendicular wall with positive surgical margins. Chemotherapy was proposed with a good response. Our patient is free from disease 9 months later. CONCLUSIONS: We aimed through this case report to discuss mesenteric presentation monophasic SS, mimicking ovarian malignancy, emphasizing clinicopathological features and differential diagnoses.

2.
Am J Mens Health ; 17(2): 15579883231159343, 2023.
Article En | MEDLINE | ID: mdl-36864684

The sarcoid-like reaction is a rare autoinflammatory disease that can affect lymph nodes or organs but does not meet the diagnostic criteria for systemic sarcoidosis. Several drug classes have been associated with the development of a systemic sarcoid-like reaction, which defines drug-induced sarcoidosis-like reactions and can affect a single organ. Anti-CD20 antibodies (rituximab) have rarely been reported as responsible for this reaction and this adverse effect has mainly been described during the treatment of Hodgkin's lymphoma. We report a unique case of a sarcoid-like reaction complicating rituximab following the treatment of a mantle cell lymphoma and interesting only the kidney. The 60-year-old patient presented with severe acute renal failure 6 months after the end of his r-CHOP protocol and the urgent renal biopsy revealed acute interstitial nephritis rich in granulomas without caseous necrosis. After ruling out other causes of granulomatous nephritis, a sarcoid-like reaction was retained since infiltration was limited to the kidney. The temporal relationship between rituximab administration and the sarcoid-like reaction onset in our patient supported the diagnosis of a rituximab-induced sarcoidosis-like reaction. Oral corticosteroid treatment led to rapid and lasting improvement in renal function. Clinicians should be warned of this adverse effect and regular and prolonged monitoring of renal function should be recommended during the follow-up of patients after the end of treatment with rituximab.


Drug-Related Side Effects and Adverse Reactions , Lymphoma , Nephritis, Interstitial , Sarcoidosis , Male , Humans , Adult , Middle Aged , Rituximab/adverse effects , Kidney/physiology , Sarcoidosis/chemically induced , Sarcoidosis/drug therapy
3.
Br J Clin Pharmacol ; 89(5): 1682-1685, 2023 05.
Article En | MEDLINE | ID: mdl-36480744

AIMS: Interferon-beta (IFNß), the most widely prescribed medication for multiple sclerosis, is generally considered safe. Nevertheless, rare serious and/or life-threatening side effects have been reported such as thrombotic microangiopathy. A few mechanisms have been proposed to explain how interferon causes thrombotic microangiopathy, but immunological studies have been unable to pin this phenomenon down to a single pathophysiologic pathway. The aim of this article was to report a new mechanism explaining Interferon beta related thrombotic microangiopathy. METHODS: We report thrombotic microangiopathy in a 28-year-old male receiving interferon-beta treatment for multiple sclerosis. RESULTS: After three years of starting interferon beta therapy, the patient presented with malignant hypertension causing seizures, rapidly progressive renal failure requiring haemodialysis and haemolytic anaemia. Corticosteroid and plasma exchange sessions permitted haemolysis control. Nonetheless, the patient remained hemodialysis-dependent. Exploration of the complement system found a complement factor I deficiency whose activity normalized at the control carried out after 2 years. CONCLUSION: IFNß treatment may cause complement factor I deficit, which can lead to thrombotic microangiopathy and severe renal failure.


Multiple Sclerosis , Renal Insufficiency , Thrombotic Microangiopathies , Male , Humans , Adult , Interferon-beta/adverse effects , Multiple Sclerosis/drug therapy , Thrombotic Microangiopathies/chemically induced , Renal Insufficiency/complications
4.
Am J Mens Health ; 16(6): 15579883221139914, 2022.
Article En | MEDLINE | ID: mdl-36484293

Acute interstitial nephritis (AIN) is a relevant cause of acute renal failure. Drugs are the predominant cause, followed by infections and idiopathic lesions. AIN, as a form of hypersensitivity reaction, is an uncommon manifestation in the setting of human parasitic infections. We report a case of a polyparasitic infection (Giardia lamblia, Entamoeba coli, and Endolimax nana) resulting in a severe biopsy-proven AIN in a 61-year-old male patient. Despite the antiparasitic treatment followed by corticosteroid therapy, and during the 6-month follow-up period, the patient remained dialysis-dependent, and he developed autoimmune hemolytic anemia. Extensive search for another infection or neoplasia was negative. Immunological tests were also negative. The resulting hypersensitivity reaction to the triple parasite infection would have led to fatal evolution for the kidneys affected by this unusual type of AIN.


Anemia, Hemolytic , Dermatitis , Nephritis, Interstitial , Male , Humans , Middle Aged , Nephritis, Interstitial/diagnosis
5.
Tunis Med ; 100(7): 534-540, 2022.
Article En | MEDLINE | ID: mdl-36571742

INTRODUCTION: Colorectal cancer is a major public health problem. In younger patients, its incidence continues to rise and its prognosis appears to be worse. Its treatment is based on curative surgery associated with neo-adjuvant and adjuvant therapies. AIM: To describe the clinical and pathological characteristics of colorectal cancers in young patients. METHODS: In this monocentric cohort study, we retrospectively analyzed the clinicopathological features in colorectal cancer patients who underwent treatment from 2002 to 2014. Data of younger (group A, ≤50years) vs older (group B, >50years) patients were compared. RESULTS: Two hundred and sixty-six patients met inclusion and non-inclusion criteria. The younger and older groups consisted respectively of 25.2% and 74.8% of patients. Both groups were comparable regarding the symptom presentation and duration. Synchronous tumors were more frequent amongst the group A (10.7% vs 1.0%, p = 0.024). Preoperative staging showed a higher frequency of tumors classified as advanced stage (stages III and IV) in the group A (p = 0.001). The patients of group A were diagnosed with a higher proportion of poorly differentiated or undifferentiated adenocarcinomas (13.4% vs 3.5%, p = 0.005), the mucinous character was also more frequent in the group A (28.4%). According to the pTNM (tumor, nodes and metastases) classification, tumors were more advanced in the group A than in group B (80.6% vs 48.7%, p <0.001). CONCLUSION: This study revealed that colorectal adenocarcinomas in the younger patients, compared to the older ones, were more aggressive with a higher proportion of poorly differentiated or undifferentiated adenocarcinomas, more often mucin production and more advanced tumors.


Adenocarcinoma , Colorectal Neoplasms , Humans , Retrospective Studies , Cohort Studies , Colorectal Neoplasms/diagnosis , Colorectal Neoplasms/epidemiology , Colorectal Neoplasms/therapy , Adenocarcinoma/epidemiology , Adenocarcinoma/therapy , Adenocarcinoma/diagnosis , Prognosis , Neoplasm Staging
6.
Tunis Med ; 100(7): 561-563, 2022.
Article En | MEDLINE | ID: mdl-36571746

INTRODUCTION: Langerhans cell histiocytosis (LCH) is a rare systemic disease characterized by the abnormal overproduction of histiocytes that tend to infiltrate single or multiple organ systems leading to significant tissue damage. Although LCH can involve various organs including bone, skin, and lymph nodes, multisystem involvement of LCH is rare in adults. CASE PRESENTATION: We report the case of a 31-year-old man with LCH involving his lungs and bone. The initial radio-clinical presentation was rather in favor of pulmonary tuberculosis. Through this observation we draw attention to this rare pathology and we discuss the diagnostic elements and the therapeutic management of this pathology. CONCLUSION: Although it is occasionally difficult to discriminate LCH from other disorders, systemic evaluation might be helpful for differential diagnosis. As LCH isn't infiltrating malignant cells, strong chemotherapy regimen is not recommended in order to avoid severe toxic and side effects.


Histiocytosis, Langerhans-Cell , Tuberculosis, Pulmonary , Male , Humans , Adult , Histiocytosis, Langerhans-Cell/complications , Histiocytosis, Langerhans-Cell/diagnosis , Histiocytosis, Langerhans-Cell/drug therapy , Skin , Tuberculosis, Pulmonary/diagnosis , Diagnosis, Differential , Lung/pathology
7.
Br J Clin Pharmacol ; 2022 Nov 17.
Article En | MEDLINE | ID: mdl-36394376

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute, life-threatening and rare severe cutaneous adverse reactions induced by drugs in most cases. The drugs most often reported to be implicated in inducing TEN/SJS are allopurinol, antibacterial sulfonamides, antiepileptic drugs and oxicam. Pristinamycin is an oral streptogramin antibiotic with bactericidal activity against Gram-positive bacteria that is rarely linked to TEN. Typically, this condition develops 4-28 days after drug exposure, Herein, we report a case of a 71-year-old female who developed TEN within 3 days of administration of pristinamycin and was managed successfully with supportive care, including intravenous fluids, pain control, prophylactic antibiotics and intravenous methylprednisolone. This case of rapidly developing SJS/TEN after administration of pristinamycin highlights the possibility that these complications can develop within only a few days following ingestion of drugs thought to be probably safe.

8.
Pan Afr Med J ; 42: 30, 2022.
Article Fr | MEDLINE | ID: mdl-35910055

Triple-negative breast cancer (TNBC) is a very heterogeneous disease and refers to a subgroup of breast cancer. The purpose of this study was to describe the clinical, pathological and molecular features of TNBC. We conducted a retrospective analysis of 56 cases of TNBC diagnosed in the Department of Pathology at the Farhat Hached University Hospital over a period of three years (2018-2020). The incidence of TNBC was 5.62% and the mean age of the patients was 50.36 years. The most frequent reason for consultation was the discovery of breast nodules (98.21%). The diagnosis of TNBC was confirmed in 24 cases (42.86%) based on the analysis of mastectomy specimens. Invasive carcinoma of no special type (NST) was the most common histological type (47 cases; 82.5%). Mean tumor size was 35.5 mm. SBR grading was only specified in 42 cases (73.68%). Grade III was the most predominant type (78.6%, n = 33). The notion of lymph node metastasis was found in 44 reports and lymph node invasion was reported in 19 patients (43.2%). Immunohistochemical study showed that all patients were hormone receptor-negative (RO and RP) with no Her2 overexpression (n = 56; 100). This study results are generally in agreement with literature data. Despite recent molecular classifications, there is no clinically verified diagnostic test allowing for clear therapeutic standardization.


Breast Neoplasms , Triple Negative Breast Neoplasms , Breast Neoplasms/diagnosis , Breast Neoplasms/epidemiology , Breast Neoplasms/pathology , Female , Humans , Lymphatic Metastasis , Mastectomy , Middle Aged , Prognosis , Receptors, Progesterone , Retrospective Studies , Triple Negative Breast Neoplasms/diagnosis , Triple Negative Breast Neoplasms/epidemiology , Triple Negative Breast Neoplasms/pathology
9.
Case Rep Med ; 2022: 5131705, 2022.
Article En | MEDLINE | ID: mdl-35813004

Introduction: Sister Mary Joseph's nodule (SMJN) is a rare illness characterized by an umbilical mass caused by tumor metastases in the abdomen or pelvis. The most common main site of SMJN in women is ovarian cancer. Case Presentation. A 73-year-old woman with no pathological history came to our emergency room with a one-month history of umbilicus enlargement. A 9-centimeter uncomfortable umbilical swelling with hard consistency was discovered during a clinical examination. An ovarian tumor with several local expansions was seen on an abdominal CT scan. It was linked to peritoneal metastases, one of which extends via a supraumbilical hernial orifice and into intestinal tissues in the same hernia sac. The umbilical tumor was removed from the patient. A moderately differentiated serous carcinoma with ovarian origin was identified in a periumbilical site on histological testing. Conclusion: The presence of an SMJN is a rare but significant issue that clinicians must examine because it is associated with a bad prognosis. Early detection and diagnosis of the original lesion can lead to more effective treatment and a higher rate of survival.

10.
Ann Biol Clin (Paris) ; 80(3): 233-243, 2022 06 30.
Article En | MEDLINE | ID: mdl-35796474

Although the significance of DNA mismatch repair (MMR) protein expression in colorectal cancer is well-established, it remains contentious in extra-colorectal cancers and mainly in gastric adenocarcinoma. Data from Africa and Arab world remain limited. This study explored the MMR expression in gastric adenocarcinoma and evaluated its clinicopathological and prognostic signification among Tunisian patients. A retrospective study of 72 gastric adenocarcinomas was carried out. Clinicopathological particularities and patient outcomes were recorded. MMR expression was determined by immunohistochemistry on whole sections of archived material. Survival analysis was realized utilizing the Kaplan-Meier estimates and Log-Rank test. Expression of MMR proteins was observed in 84.7% of gastric adenocarcinoma samples. The 11 remaining samples (15.3%) exhibited an altered pattern of MMR protein. A significant association was identified between deficient MMR expression and advanced age (p = 0.03), intestinal type (p = 0.04) and lymph node metastases (p = 0.04). No other significant relationship was observed with the remaining selected tumor features. Patient survival was significantly associated with lymph node invasion (p = 0.002), distant metastases (p = 0.02) and tumor differentiation (p = 0.03), but not with MMR status (p = 0.83). MMR deficiency was related to advanced-age, intestinal type and nodal metastasis, but not to survival of Tunisian patients with gastric adenocarcinoma. Larger multicenter studies with additional molecular investigation are required to more explore these tumors.


Bien que l'importance de l'expression des protéines de réparation des mésappariements de l'ADN (MMR) dans le cancer colorectal soit bien établie, elle reste controversée dans les cancers extra-colorectaux et principalement dans l'adénocarcinome gastrique. Les données de l'Afrique et du monde arabe restent limitées. Cette étude a exploré l'expression des protéines MMR dans l'adénocarcinome gastrique et a évalué sa signification clinicopathologique et pronostique chez les patients tunisiens. Une étude rétrospective de 72 adénocarcinomes gastriques a été réalisée. Les particularités clinicopathologiques et pronostiques des patients ont été enregistrées. L'expression des protéines MMR a été déterminée par immunohistochimie. L'analyse de survie a été réalisée en utilisant la méthode de Kaplan-Meier et le test Log-Rank. L'expression des protéines MMR a été observée dans 84,7 % des échantillons d'adénocarcinome gastrique. Les 11 cas restants (15,3 %) présentaient un profil d'expression altérée des protéines MMR. Une association significative a été identifiée entre l'expression déficiente de MMR et l'âge avancé (p = 0,03), le type intestinal (p = 0,04) et les métastases ganglionnaires (p = 0,04). Aucune autre relation significative n'a été observée avec les autres caractéristiques tumorales sélectionnées. La survie des patients était significativement associée à l'envahissement des ganglions lymphatiques (Log Rank, p = 0,002), aux métastases à distance (Log Rank, p = 0,02) et à la différenciation tumorale (Log Rank, p = 0,03), mais pas à l'expression de MMR (Log Rank, p = 0,03). Rang, p = 0,83). Le déficit de l'expression des protéines MMR était lié à l'âge avancé, au type intestinal et aux métastases ganglionnaires, mais pas à la survie des patients tunisiens ayant un adénocarcinome gastrique. Des études multicentriques avec des investigations moléculaires supplémentaires sont nécessaires pour explorer davantage le cancer gastrique avec expression déficiente des protéines MMR.


Adenocarcinoma , Stomach Neoplasms , Adenocarcinoma/diagnosis , Adenocarcinoma/genetics , DNA , DNA Mismatch Repair , Humans , Prognosis , Retrospective Studies , Stomach Neoplasms/diagnosis , Stomach Neoplasms/genetics
11.
Clin Case Rep ; 10(4): e05674, 2022 Apr.
Article En | MEDLINE | ID: mdl-35414911

Pityriasis rosea is a common, acute, self limiting inflammatory skin disease. Pityriasis rosea-like eruptions (PR-LE) have been reported after drugs. The clinical presentation of PR-LE can be distinguished from pityriasis rosea. We reporte a 41-year-old woman who developed PR-LE 5 days after administration domperidone.

12.
Int J Surg Case Rep ; 85: 106271, 2021 Aug.
Article En | MEDLINE | ID: mdl-34388901

INTRODUCTION: Dacryops is a rare benign cystic lesion of the lacrimal gland often developing in the palpebral gland. The exact etiology of dacryops remains unclear. Diagnosis could be suspected clinically and established histopathologically. Treatment is commonly surgical. PRESENTATION OF CASE: We report the case of a 75-year-old woman with past history of total conservative parotidectomy for a benign tumor 10 years ago. She presented to the Ophtalmology Department at Farhat Hached University Hospital of Sousse with a painful swelling of the left supero-external orbital angle. The patient underwent complete excision of the cyst using the conjunctival approach. Histopathological examination showed characteristic features of dacryops with foci of chronic inflammatory cell infiltrates. DISCUSSION: Dacryops is commonly seen in young adults or middle-aged individuals with a slight female preponderance. In some cases, cystic enlargement of dacryops can lead to mechanical ptosis, dystopia, limitation of extraocular movements, diplopia, and, in rare cases, blepharoptosis. However in this case, the patient was an elderly woman presenting a painless mass without ptosis. As in our case, excision using the conjunctival approach under microscope has excellent results without complications. Histopathological examination confirms the diagnosis and helps to rule out differential diagnoses. CONCLUSION: As reported in the present case, histopathological examination confirms clinically recognized or suspected dacryops. If untreated, dacryops may lead to ptosis, proptosis and/or dystopia. Complete excision of the cyst is often curative.

14.
Technol Health Care ; 29(3): 431-443, 2021.
Article En | MEDLINE | ID: mdl-33285655

BACKGROUND: Thyroid metabolism involves iodine, which allows us to use radioactive iodine for diagnostic and therapy purposes. The efficiency of radioiodine therapy depends on several parameters; the ability of thyroid tissue to uptake radioactive iodine is one of them. OBJECTIVE: The objective of this work is to quantify the radioactive iodine uptake on thyroid tissue. METHODS: In this work, we developed a method to quantify the in vivo uptake of iodine-131 on sections of thyroid glands removed by thyroidectomies. We performed an analysis of histological sections of the thyroid tissue by beta imaging. We had the opportunity to quantify the fixed radioactivity and to analyze its distribution in the thyroid gland, thanks to the good spatial resolution available with the type of detector used. RESULTS: The results gave a high image resolution showing the heterogeneity of iodine-131 fixation by the thyroid tissue. We were able to quantify the tissue radioactivity in mega Becquerel (MBq) per volume unit. CONCLUSION: This work has shown that the direct quantification of the thyroid tissue uptake is possible using the beta imaging system.


Iodine , Thyroid Neoplasms , Humans , Iodides , Iodine Radioisotopes
17.
Int J Surg Case Rep ; 64: 133-138, 2019.
Article En | MEDLINE | ID: mdl-31655282

INTRODUCTION: Metastatic tumors to the gallbladder are uncommon. Metastases from renal cell carcinoma (RCC) to the gallbladder are exceptional. Frequencies of less than 0.6% reported in large autopsy reviews and few cases have been reported in the literature. PRESENTATION OF CASE: Herein we present a case of a 50-year-old man that developed four years after radical nephrectomy for RCC, a gallbladder metastasis, discovered incidentally on Computed Tomography (CT) scan. It was described as an intraluminal gallbladder polyp. Radiological features were very suggestive of primary gallbladder carcinoma, thus the patient had a laparoscopic cholecystectomy. The pathological examination of the surgical specimen concluded to a RCC metastasis. Immunochemistry with vimentin and pancytokeratin were supportive of this diagnosis. The clinical course was uneventful after 18 months period of follow up. DISCUSSION: At the time of presentation, almost one-third of the patients with RCC are metastatic. Metastases to the gallbladder are extremely rare. Clinical presentation and physical examination are unspecific. Radiological findings can raise information and orient the diagnosis although the difference between both diagnoses remains difficult. Usually, it is a pedunculated and not associated with gallstones with enhancement on CT scan. The treatment remains surgical with R0 cholecystectomy, and prognosis is mainly related to disease free interval and single site metastasis. CONCLUSION: We highlight here the challenge to make the difference between a primary gallbladder carcinoma and metastasis from RCC. Diagnosis is made on pathological examination and immunochemistry.

18.
Ann Biol Clin (Paris) ; 77(3): 307-317, 2019 Jun 01.
Article En | MEDLINE | ID: mdl-31131831

The study investigated the pattern of MGMT promoter methylation and the expression of MGMT, P53, EGFR, MDM2 and PTEN proteins in glioblastomas multiforme (GBM) and evaluated their prognostic significance. We carried out a retrospective study of 80 GBM. Expression of MGMT as well as of P53, EGFR, MDM2 and PTEN was investigated by immunohistochemistry. MGMT promoter methylation was investigated by methylation specific-PCR of bisulfite-treated DNA. Twenty-five GBM exhibited MGMT expression. Methylation of MGMT promoter was detected in 35.1% of cases. No significant concordance was reported between MGMT promoter methylation and protein expression (κ=-0.047, p=0.11). MGMT promoter methylation was significantly associated only with PTEN expression (p=0.001): no other significant association was identified with clinical parameters as well as with expression of P53, EGFR and MDM2 (p >0.05). Tumor recurrence was significantly associated with unmethylated MGMT promoter (p=0.01) but not with MGMT expression (p=0.51). Recurrence-free survival (RFS) was significantly better among patients with methylated MGMT promoter (log rank, p <0.0001) and PTEN expression (log rank, p=0.025) but not with MGMT expression (log rank, p=0.308). As well, using univariate analysis, MGMT promoter methylation (p=0.001) and PTEN expression (p=0.044) were significantly associated with RFS. In multivariate analysis, only MGMT promoter methylation was significantly associated with RFS (p=0.003). Together, our findings support that MGMT protein expression doesn't reflect the MGMT promoter methylation status. Furthermore, MGMT promoter methylation remains a useful prognostic marker in Tunisian patients with GBM. PTEN expression could be a potential prognostic marker of this tumor.


Brain Neoplasms/diagnosis , DNA Modification Methylases/genetics , DNA Modification Methylases/metabolism , DNA Repair Enzymes/genetics , DNA Repair Enzymes/metabolism , Glioblastoma/diagnosis , Tumor Suppressor Proteins/genetics , Tumor Suppressor Proteins/metabolism , Adolescent , Adult , Aged , Brain Neoplasms/genetics , Brain Neoplasms/metabolism , Child , Child, Preschool , DNA Methylation , DNA Modification Methylases/analysis , DNA Repair Enzymes/analysis , ErbB Receptors/metabolism , Female , Gene Expression Regulation, Neoplastic , Glioblastoma/genetics , Glioblastoma/metabolism , Humans , Immunohistochemistry , Male , Middle Aged , PTEN Phosphohydrolase/metabolism , Predictive Value of Tests , Prognosis , Promoter Regions, Genetic , Proto-Oncogene Proteins c-mdm2/metabolism , Retrospective Studies , Tumor Suppressor Protein p53/metabolism , Tumor Suppressor Proteins/analysis , Tunisia , Young Adult
19.
Rare Tumors ; 11: 2036361318820171, 2019.
Article En | MEDLINE | ID: mdl-30719261

BACKGROUND: Soft tissue leiomyosarcomas are rare, accounting for almost 5%-10% of all soft tissue sarcomas; they account for almost 1% of all sarcomas. They are aggressive tumors where location, size, and management require a multidisciplinary approach. Since there are few series published, we here analyze epidemiological pattern, clinical and pathologic features of soft tissue leiomyosarcomas. METHODS: We conducted a retrospective study of 29 consecutive cases of histologically proven soft tissue leiomyosarcoma extracted from the database of the Cancer Registry of the Center of Tunisia and the Department of Pathology of Farhat Hached University Hospital of Sousse of Tunisia, during a 10-year period (from January 1996 to December 2005). Epidemiologic details, clinico-pathological features, and treatment modalities were assessed with focus on patients' 5-year overall survival, tumor relapse, and metastases. RESULTS: Soft tissue leiomyosarcoma accounted for 17.5% of all soft tissue sarcomas diagnosed at our pathology department. Most of patients were of advanced age (median: 52 years), with extremes ranging from 12 and 87 years. There was a slight male predominance (sex-ratio = 1.07). Tumors were located mostly in the lower limbs (45%). Deep sites as retroperitoneum was found only in two cases. Tumor size was more than 5 cm in 83% of cases (average size = 9.4 cm). Five cases had metastasis on initial staging. For 24 patients, the disease was locally limited at the moment of diagnosis. Palliative chemotherapy was indicated for four patients and surgery was performed for 20 patients. Local recurrence occurred in 11 patients (55% of operated patients) and metastasis in 6 patients. Overall, 5-year survival was about 24%. CONCLUSION: Our study results highlight the scarcity of soft tissue leiomyosarcoma. Unfortunately, unusual tumor sites, disease's advanced stages, and intralesional resection made the prognosis poorer than in other series. Clinical course of soft tissue leiomyosarcoma was highly marked by local recurrence and metastasis.

20.
J Egypt Natl Canc Inst ; 31(1): 1, 2019 Oct 22.
Article En | MEDLINE | ID: mdl-32372136

BACKGROUND: Extranodal NK/T-cell lymphomas (ENKTL) are rare non-Hodgkin's lymphomas with aggressive clinical behavior. ENKTL are frequently associated with the Epstein-Barr virus (EBV). Data on ENKTL in Africa and Arab world are extremely limited. The study investigated the clinicopathological characteristics, EBV infection, and immunophenotype of ENKTL in Tunisia. We conducted a retrospective study of ENKTL. Main clinicopathological features were reported. The expression of CD3, CD4, CD5, CD8, CD20, CD56, CD57, and Granzyme B were analyzed by immunohistochemistry. EBV infection was detected by IHC (LMP-1) and Epstein-Barr encoding region (EBER1/2) in situ hybridization. RESULTS: A total of nine ENKTL were identified (mean age of 48 years and male-to-female ratio of 8:1). There were five nasal ENKTL, and the remaining four cases had extranasal involvement (palate, sub-mandibular gland, skin, and soft tissues of the ankle). The histopathology showed a lymphoid and pleomorphic proliferation characterized by images of angiocentrism. Strong and diffuse CD3 expression was observed in all cases. Tumor cells exhibited an expression of CD5 (two cases), CD8 (three cases), CD56 (six cases), CD57 (three cases), and Granzyme B (eight cases). All ENKTL cases were EBV-associated. Overall 5-year survival rate was 57%. Although six ENKTL were diagnosed at early clinical stages, the prognosis was unfavorable and associated with patient death in three cases. CONCLUSIONS: ENKTL are exceptional in Tunisia with unfavorable outcome. Histopathological diagnosis remains challenging in clinical practice. However, a careful histopathological examination combined with a correct interpretation of immunohistochemistry and in situ hybridization results refines the ENKTL diagnosis.


Epstein-Barr Virus Infections/complications , Lymphoma, Extranodal NK-T-Cell/complications , Lymphoma, Extranodal NK-T-Cell/pathology , Adult , Aged , Antigens, Differentiation/metabolism , Epstein-Barr Virus Infections/epidemiology , Female , Herpesvirus 4, Human/isolation & purification , Humans , Lymphoma, Extranodal NK-T-Cell/epidemiology , Lymphoma, Extranodal NK-T-Cell/virology , Male , Middle Aged , Prognosis , RNA, Viral/metabolism , Retrospective Studies , Survival Rate , Tunisia/epidemiology , Viral Matrix Proteins/metabolism
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